Fatal Brain Disease in US Man Likely Came from UK Beef

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A U.S. man who developed a rare and fatal brain disease likely
got the disease from eating beef while living abroad more than a
decade earlier, according to a new report of the case.

The man, who was in his 40s when he first got sick, died in Texas
in May 2014. Because the condition, known as
variant Creutzfeldt-Jakob disease, is so rare, the man was
misdiagnosed and even hospitalized for psychiatric symptoms
multiple times before doctors suspected the true cause of his
symptoms, according to the report from researchers at the Centers
for Disease Control and Prevention and Baylor College of
Medicine.

Variant Creutzfeldt-Jakob disease is thought to arise from eating
beef contaminated with infectious prions, which are proteins that
fold abnormally and form lesions in the brain. In the United
Kingdom, there have been nearly 200 cases of variant
Creutzfeldt-Jakob disease tied to eating contaminated beef in the
1980s and 1990s. (In cattle, the condition is sometimes known as
" mad
cow disease.")

In the United States, there have been three earlier cases of
variant Creutzfeldt-Jakob disease, but in all of these cases, the
patients had previously lived in either the United Kingdom or
Saudi Arabia.

In the new case, the man had never lived in the United Kingdom,
or in any country where people have been suspected of acquiring
variant Creutzfeldt-Jakob disease (vCJD) from eating beef.
However, the man had lived in Kuwait, Russia and Lebanon, and the
researchers suspect he likely got the disease while living in one
of these countries, all of which had imported beef from the
United Kingdom.

The man's case shows "the persistent risk for vCJD acquired in
unsuspected geographic locations and highlights the need for
continued global surveillance and awareness to prevent further
dissemination of vCJD," the researchers said. [ 10
Things You Didn't Know About the Brain ]

The man was born in the Middle East and moved to the United
States in the 1990s. He became a naturalized U.S. citizen, and
was healthy until late 2012, when he started to become depressed
and anxious. He later developed hallucinations and changes in
behavior, including becoming more withdrawn and secretive.

A little over a year after his initial symptoms, the man was
hospitalized several times for his psychiatric problems. Doctors
thought the man might have a type of depression that comes with
symptoms of psychosis, or
bipolar disorder with psychosis, but treatments for these
disorders failed to improve his condition.

Around this time, the man also experienced jerky, involuntary
movements and trouble with his reflexes. Doctors ran a number of
tests to rule out autoimmune diseases, heavy metal poisoning,
vitamin deficiency and hormone disorders.

Doctors then suspected the man had vCJD, and so samples of blood,
urine and spinal fluid were sent for testing. The blood test
(which can miss cases) came back negative, but the
urine test — which was only recently developed, and is still
considered experimental — came back positive.

A brain biopsy could have confirmed the diagnosis, but by this
time, doctors were concerned that the procedure would worsen the
man's health. The man's condition declined further, and he died
shortly after that point, which was 18 months after he initially
showed symptoms.

A brain autopsy confirmed the man had vCJD.

"The potential difficulty in making the clinical diagnosis in
many patients with vCJD and the delay with which the disease is
first suspected raises the concern that vCJD can be missed," the
researchers write in the May issue of the journal Emerging
Infectious Diseases.

Although it's not clear where the man became infected, the
researchers said his risk of exposure to prions was highest when
he was living in Kuwait because of the amount of beef exported to
that country from the United Kingdom.

"This case underscores that the diagnosis of vCJD should not be
dismissed if the patient has not resided in a country with a
known endemic case of vCJD," the researchers said. "Given the
several decades' long potential incubation periods estimated from
epidemiologic modeling, the international occurrence of
additional vCJD cases can be reasonably anticipated."